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BPGM Rabbit pAb (bs-9519R)  
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產(chǎn)品編號 bs-9519R
英文名稱 BPGM Rabbit pAb
中文名稱 紅細(xì)胞2,3 - 二磷酸甘油酸合成酶抗體
別    名 2, 3-bisphosphoglycerate mutase; 2, 3-bisphosphoglycerate synthase; 3-bisphosphoglycerate mutase; 3-bisphosphoglycerate synthase; 3-diphosphoglycerate mutase; Ab2 098; AI323730; AL022789; Bisphosphoglycerate mutase; BPG dependent PGAM; BPG-dependent PGAM; Bpgm; BPGM; C86192; DPGM; Erythrocyte 2, 3 bisphosphoglycerate mutase; PMGE_HUMAN; 2, 3-bisphosphoglycerate mutase, erythrocyte; erythrocyte; 2 antibody.  
研究領(lǐng)域 腫瘤  心血管  細(xì)胞生物  信號轉(zhuǎn)導(dǎo)  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應(yīng) Human,Mouse,Rat (predicted: Rabbit,Sheep,Cow)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 30 kDa
檢測分子量
細(xì)胞定位 細(xì)胞漿 細(xì)胞外基質(zhì) 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human BPGM: 101-200/259 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 BPGM (2,3-bisphosphoglycerate mutase) is a 259 amino acid protein that belongs to the phosphoglycerate mutase family and exists as a homodimer that plays a crucial role in the regulation of hemoglobin oxygen. Specifically, BPGM catalyzes the conversion of 3-phospho-D-glyceroyl phosphate to 2,3-bisphospho-D-glycerate (2,3-BPG), a reaction that is essential for controlling the concentration of 2,3-BPG within the cell. The gene encoding BPGM maps to human chromosome 7, which houses over 1,000 genes and comprises nearly 5% of the human genome. Defects in some of the genes localized to chromosome 7 have been linked to Osteogenesis imperfecta, Williams-Beuren syndrome, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome.
Involvement in disease:
Defects in BPGM are the cause of bisphosphoglycerate mutase deficiency (BPGMD) . A disease characterized by hemolytic anemia, splenomegaly, cholelithiasis and cholecystitis.

Function:
Plays a major role in regulating hemoglobin oxygen affinity by controlling the levels of its allosteric effector 2,3-bisphosphoglycerate (2,3-BPG). Also exhibits mutase (EC 5.4.2.1) and phosphatase (EC 3.1.3.13) activities.

Subunit:
Homodimer.

Tissue Specificity:
Expressed in red blood cells. Expressed in non-erythroid cells of the placenta; present in the syncytiotrophoblast layer of the placental villi at the feto-maternal interface (at protein level).

Post-translational modifications:
Glycation of Lys-159 in diabetic patients inactivates the enzyme.

DISEASE:
Defects in BPGM are the cause of bisphosphoglycerate mutase deficiency (BPGMD) [MIM:222800]. A disease characterized by hemolytic anemia, splenomegaly, cholelithiasis and cholecystitis.

Similarity:
Belongs to the phosphoglycerate mutase family. BPG-dependent PGAM subfamily.

SWISS:
P07738

Gene ID:
669

Database links:

Entrez Gene: 669 Human

Entrez Gene: 12183 Mouse

Entrez Gene: 100009096 Rabbit

Entrez Gene: 296973 Rat

Omim: 222800 Human

SwissProt: Q3T014 Cow

SwissProt: P07738 Human

SwissProt: P15327 Mouse

SwissProt: P07952 Rabbit

Unigene: 198365 Human

Unigene: 282863 Mouse

Unigene: 204528 Rat



產(chǎn)品圖片
Sample: Lane 1: Mouse NIH/3T3 cell lysates Lane 2: Rat Placenta tissue lysates Lane 3: Rat Kidney tissue lysates Lane 4: Human Jurkat cell lysates Lane 5: Human 293T cell lysates Primary: Anti-BPGM (bs-9519R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 30 kDa Observed band size: 30 kDa
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